Study of the Cochlear Function in Patients with Myotonic Dystrophy Type 1

Autor: Dimitrios G. Balatsouras, Stavros Korres, Marios Panas, Dimitrios Felekis, John Xenelis
Rok vydání: 2013
Předmět:
Zdroj: Otolaryngology–Head and Neck Surgery. 149
ISSN: 1097-6817
0194-5998
Popis: Objectives:Myotonic Dystrophy type 1 is a progressive autosomal dominant multisystem disorder, the second most common form of muscular dystrophy. The disease is associated with various vestibular, oculomotor, and auditory disorders. The aim of the present study was to undertake a detailed vestibular and audiological evaluation, and specifically to analyze the cochlear function using transiently evoked otoacoustic emissions.Methods:24 patients with genetically diagnosed myotonic dystrophy type 1 were studied during the past 4 years in Eginition hospital of Athens. Twenty-one normal subjects were used as controls. Testing included pure-tone audiometry, tympanometry, auditory brainstem responses (Abr), transiently evoked otoacoustic emissions, and nystagmography.Results:Tympanograms were normal in most patients. 15 (62.5%) patients had a varying degree of sensorineural hearing loss, with no conductive element. In 9 of them (37.5%), ABR abnormalities were found, such as absence of wave I or increased latencie...
Databáze: OpenAIRE